Associated rare anomalies in prune belly syndrome: A case report

The triad of deficient abdominal wall musculature, undescended testes and urinary tract anomalies characterizes the Prune Belly Syndrome (PBS). PBS can be associated with other comorbid urological and non urological conditions. But the full pathogenesis and best treatment is still a matter of debate...

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Main Author: Andreas Fette (Author)
Format: Book
Published: Elsevier, 2015-02-01T00:00:00Z.
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Summary:The triad of deficient abdominal wall musculature, undescended testes and urinary tract anomalies characterizes the Prune Belly Syndrome (PBS). PBS can be associated with other comorbid urological and non urological conditions. But the full pathogenesis and best treatment is still a matter of debate. A term newborn with a classical PBS (Woodhouse Group 2, Smith and Woodard Group 2) plus lung hypoplasia and funnel chest deformity, a megapenis with a tight phimosis and an obturated anterior urethra is presented. Unfortunately, the baby died in urosepsis and renal failure in his 3rd week of life, despite urine drainage surgery and peritoneal dialysis undertaken. According to the best of our knowledge, this is an unique combination of rare anomalies in PBS patients.
Item Description:2213-5766
10.1016/j.epsc.2014.12.007